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Mice expressing HLA-DQ6alpha8beta transgenes develop polychondritis spontaneously
Jennifer L Lamoureux1, Jane Hoyt Buckner, Chella S David
1Department of Microbiology and Immunology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, North Dakota, USA.
Arthritis Research & Therapy
|July 29, 2006
Summary
Transgenic mice develop spontaneous polychondritis (SP), a relapsing autoimmune disease affecting cartilage. This new model mimics human relapsing polychondritis (RP) without a strong collagen-specific immune response, aiding disease mechanism research.
Area of Science:
- Immunology
- Autoimmune Diseases
- Rheumatology
Background:
- Relapsing polychondritis (RP) is a rare autoimmune disorder characterized by recurrent inflammation and destruction of cartilage.
- Previous research established a collagen-induced polychondritis model in transgenic mice.
Purpose of the Study:
- To investigate the development of spontaneous polychondritis (SP) in HLA-DQ6alphabeta 8alphabeta transgenic mice.
- To characterize the clinical and immunological features of SP and compare them to human RP and collagen-induced polychondritis.
Main Methods:
- Development and observation of spontaneous polychondritis in HLA-DQ6alphabeta 8alphabeta transgenic mice.
- Clinical assessment of affected sites (ears, nose, joints) and histopathological analysis of cartilage.
- Analysis of serum immunoglobulin levels and collagen-specific immune responses.
Main Results:
- Transgenic mice developed spontaneous polychondritis (SP) with polyarthritis, auricular chondritis, and nasal chondritis.
- Auricular chondritis in SP exhibited a relapsing/remitting pattern, similar to human RP.
- SP showed elevated total IgG but lacked a strong collagen type II-specific immune response, and had a female preponderance.
Conclusions:
- Spontaneous polychondritis in these transgenic mice serves as a valuable model for studying RP pathogenesis.
- This model allows investigation of polychondritis mechanisms independent of a strong collagen-specific immune response.
- The SP model offers insights into the initiatory events and immunopathogenic mechanisms of cyclic cartilage inflammation.